絞り込み

16525

広告

ウマが人の行動を認識するのは「声」と「表情」の関連から (NHK)

移動の足などとして数千年前から家畜化が進んだウマは、人の声と表情の2つを関連づけて人の行動を認識していることが北海道大学などの実験でわかりました。身近な動物が人...

  1. 医療保険より100倍マシ! 学資保険の選...
  2. 食べられない高齢者にはこう介入する
  3. 血友病Aに週1回皮下注射の抗体医薬登場
  4. 39歳女性。胃痛、胸焼け

ニュース一覧

D409H GBA1 mutation accelerates the progression of pathology in A53T α-synuclein transgenic mouse model.

著者 Kim D , Hwang H , Choi S , Kwon SH , Lee S , Park JH , Kim S , Ko HS
Acta Neuropathol Commun.2018 Apr 27 ; 6(1):32.
この記事をPubMed上で見るPubMedで表示
この記事をGoogle翻訳上で見る Google翻訳で開く

スターを付ける スターを付ける     (27view , 0users)

Full Text Sources

Miscellaneous

Heterozygous mutations in glucocerebrosidase 1 (GBA1) are a major genetic risk factor for Parkinson's disease and Dementia with Lewy bodies. Mutations in GBA1 leads to GBA1 enzyme deficiency, and GBA1-associated parkinsonism has an earlier age of onset and more progressive parkinsonism. To investigate a potential influence of GBA1 deficiency caused by mutations in GBA1 on the disease progression of PD, GBA1 mice carrying D409H knock-in mutation were crossbred with the human A53T (hA53T) α-synuclein transgenic mice. Here, we show that GBA1 enzyme activity plays a significant role in the hA53T α-synuclein induced α-synucleinopathy. The expression of D409H GBA1 markedly shortens the lifespan of hA53T α-synuclein transgenic mice. Moreover, D409H GBA1 expression exacerbates the formation of insoluble aggregates of α-synuclein, glial activation, neuronal degeneration, and motor abnormalities in the hA53T α-synuclein transgenic mice. Interestingly, the expression of D409H GBA1 results in the loss of dopaminergic neurons in the substantia nigra pars compacta of hA53T transgenic mice. Taken together, these results indicate that GBA1 deficiency due to D409H mutation affects the disease onset and course in hA53T α-synuclein transgenic mice. Therefore, strategies aimed to maintain GBA1 enzyme activity could be employed to develop an effective novel therapy for GBA1 linked-PD and related α-synucleinopathies.
PMID: 29703245 [PubMed - in process]
印刷用ページを開く Endnote用テキストダウンロード